A retrospective study of bosentan in pulmonary arterial hypertension associated with congenital heart disease.

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dc.contributor.authorDurongpisitkul, Kritvikromen_US
dc.contributor.authorJakrapanichakul, Dechoen_US
dc.contributor.authorSompradikul, Sureeen_US
dc.date.accessioned2009-05-27T18:37:15Z
dc.date.available2009-05-27T18:37:15Z
dc.date.issued2008-02-09en_US
dc.descriptionChotmaihet Thangphaet.en_US
dc.description.abstractBACKGROUND: Pulmonary Arterial Hypertension (PAH) plays a significant role in morbidity and mortality of patients with congenital heart disease (CHD). Bosentan, a dual endothelin receptor antagonist has been approved for PAH patients with Eisenmenger physiology (EP). The authors retrospectively reviewed the efficacy and safety of bosentan in Thai PAH patients associated with CHD. MATERIAL AND METHOD: The study population was obtained from the databases of the CHD patients at Siriraj Hospital from October 2004 to April 2007 who received 6 months of bosentan treatment. Inclusion criteria are: CHD with Eisenmenger physiology (EP) or those with severe PAH after surgical repair or interventional cardiac catheterization. Clinical characteristics including the 6-- minute walk test (6MWT) distances, oxygen saturation (O2 sat), New York Heart Association (NYHA) functional class, and right ventricular systolic pressure (RVSP) at baseline were compared with those at 1, 3, and 6 months post bosentan treatment. Signs and symptoms of adverse events were also recorded. RESULTS: There were 11 patients from among those who fitted the inclusion criteria and whose records were examined. Their average age was 51.1 +/- 10.1 years old (13-61 years old). Patients were divided into 2 groups; Group A (6 patients) was PAH with EP and Group B (5 patients) was PAH post intervention. In group A, the 6MWT increased from 151 +/- 69 meters to 293 +/- 61 meters (p = 0.001) with the average increase of 38 +/- 61 meters. The 2O sat increased from 83 +/- 12.7% to 91.8 +/- 5.6% (p = 0.038) with an average increase of 1.4 +/- 0.07%. There was no significant change in right ventricular systolic pressure (RVSP). In group B, there was a trend in 6MWT improvement from 274 +/- 69 meters to 312 +/- 38 meters but this was not statistically different. There were improvements in the NYHA functional class in both groups. There was no significant increase in serum aminotransferase at the end of 6 months in each patient. CONCLUSION: There are benefits of bosentan for treatment of severe PAH in CHD, especially in patients with Eisenmenger physiology. Obvious benefits are an improvement of 6MWT and O2 sat.en_US
dc.description.affiliationDivision of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand. sikdr@mahidol.ac.then_US
dc.identifier.citationDurongpisitkul K, Jakrapanichakul D, Sompradikul S. A retrospective study of bosentan in pulmonary arterial hypertension associated with congenital heart disease. Journal of the Medical Association of Thailand. 2008 Feb; 91(2): 196-202en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/39515
dc.language.isoengen_US
dc.source.urihttps://www.mat.or.th/journal/all.phpen_US
dc.subject.meshAdolescenten_US
dc.subject.meshAdulten_US
dc.subject.meshAntihypertensive Agents --therapeutic useen_US
dc.subject.meshDatabases as Topicen_US
dc.subject.meshEisenmenger Complexen_US
dc.subject.meshEndothelins --drug effectsen_US
dc.subject.meshExercise Testen_US
dc.subject.meshFemaleen_US
dc.subject.meshHealth Statusen_US
dc.subject.meshHealth Status Indicatorsen_US
dc.subject.meshHeart Defects, Congenital --complicationsen_US
dc.subject.meshHeart Ventriclesen_US
dc.subject.meshHumansen_US
dc.subject.meshHypertension, Pulmonary --drug therapyen_US
dc.subject.meshMaleen_US
dc.subject.meshMiddle Ageden_US
dc.subject.meshPulmonary Artery --drug effectsen_US
dc.subject.meshRetrospective Studiesen_US
dc.subject.meshSulfonamides --therapeutic useen_US
dc.subject.meshSystoleen_US
dc.subject.meshTime Factorsen_US
dc.subject.meshTreatment Outcomeen_US
dc.titleA retrospective study of bosentan in pulmonary arterial hypertension associated with congenital heart disease.en_US
dc.typeJournal Articleen_US
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